244 2009; 11 (3) CJEM • JCMU

ABSTRACT

Erythroderma is a potentially fatal dermatologic emergencythat is often mistaken for infection. Indeed, the fact that it isdifficult to diagnosis is the main contributor to its significantmortality rate, as treatment is readily available. We present acase of a 36-year-old man who was incorrectly diagnosed andtreated for 2 months. We review the etiologies, initial work-upand management of this disease. In our case, the patient wasill, had lost 11.3 kg and developed systemic inflammatory re-sponse syndrome. Without proper treatment he was at risk ofdeveloping full-blown sepsis. Although there are manycauses of erythroderma, prompt initial treatment directed atthe underlying etiology typically results in a rapid remission.

RÉSUMÉ

L’érythrodermie constitue une urgence dermatologique po-tentiellement fatale qui peut souvent être confondue avecune infection. De fait, on attribue principalement l’importanttaux de mortalité associé à la maladie au fait qu’elle est diffi-cile à diagnostiquer, alors qu’en soi, elle se traite facilement.Nous présentons ici le cas d’un homme de 36 ans qui a reçuun traitement inadéquat pendant deux mois pour un diag-nostic erroné. Nous passons en revue les étiologies, lesanalyses et la prise en charge initiales de cette maladie. Dansce cas-ci, le patient était mal en point, il avait perdu 11,3 kget présentait un syndrome de réponse inflammatoire sys-témique. Faute de traitement approprié, il se trouvait exposéà un risque de sepsis avéré. Bien qu’il y ait de nombreusescauses de l’érythrodermie, un traitement initial instauré sansdélai et selon l’étiologie sous-jacente procure généralementune rémission rapide.

(e.g., cutaneous T-cell lymphoma and paraneoplasticphenomena), pre-existing dermatoses (e.g., atopic der-matitis, psoriasis, seborrheic dermatitis and pityriasisrubra pilaris), drug reactions, bullous disorders and al-lergic contact dermatitis. In up to 25% of cases, no un-derlying cause is found, and so it is termed idiopathicerythroderma.1

Erythroderma accounts for 1% of all dermatologicadmissions to hospital and is more commonly seen inmale patients. The importance of this disease is high-lighted by the consequences of losing the cutaneousbarrier: those affected are prone to lose heat, water, pro-tein and electrolytes, and they are more susceptible tocutaneous infections.2 The disease can be mistaken as aninfectious process and treated as such. The purpose ofthis case report is to highlight the pertinent clinicalfindings of erythroderma and its differentiation from in-fectious or malignant etiologies.

CASE REPORT

A 36-year-old man presented to our emergency depart-ment (ED). His chief complaint was a generalized pru-ritic rash ongoing for approximately 2 months. He alsocomplained of concomitant fevers, night sweats andgeneralized malaise. Over the course of the previous 2 months he had seen his general practitioner and beento a walk-in clinic. Both times, he was given diphenhy-dramine hydrochloride and a topical corticosteroidwithout much relief. Ten days before his ED visit, hehad also been treated with intravenous cefazolin for 2 days, and then switched to oral cloxacillin. He stoppedtaking the oral cloxacillin after 2 days as he felt it wasnot helping. He was prescribed oral prednisone, butonly took a 4-day course, stopping it because it also ap-peared to be ineffective.

On the day of his presentation to our ED, in addition

Erythroderma: a dermatologic emergency

Tony F. Bruno, MSc, MD;* Parbeer Grewal, MD†

This article has been peer reviewed.

CJEM 2009;11(3):244-6

Submitted Aug. 9, 2008; Revised Dec. 4, 2008; Accepted Dec. 23, 2008

From the Departments of *Family Medicine and †Dermatology, University of Alberta Hospital, Edmonton, Alta.

Keywords: erythroderma, exfoliative dermatitis, red man syndrome

CASE REPORT • RAPPORT DE CAS

INTRODUCTION

Erythroderma, also known as the red man syndrome oras exfoliative dermatitis, is defined as a generalized red-ness and scaling of the skin. It is a clinical manifestationof a variety of underlying diseases, including malignancy

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CJEM • JCMU 2009; 11 (3) 245

Erythroderma: a dermatologic emergency

to the pruritic rash, he continued to complain of fevers,profuse sweating and generalized skin desquamation.He denied any history of travel outside of Canada, ex-posure to farms, recent illness or history of skin diseasein general. He worked as an underground diamondminer, and was exposed to a variety of chemicals andoils, although he denied exposure to any new chemicalsat home or in the workplace. He was not taking anyother medications. He also denied any significant med-ical or surgical history.

On physical examination, all vital signs were normalexcept for tachycardia at 130 beats/min. He wasafebrile, although extremely warm to touch. Perspira-tion soaked through his clothes and bed linens multi-ple times while he was in the ED. His temperature wasmeasured repeatedly, as his normothermic tempera-ture appeared unlikely given such impressive exother-mia. He looked unwell, but nontoxic. His examinationwas otherwise unremarkable except for the rash. It was

a symmetric, generalized, total body, erythematousrash with prominent scaling and desquamation, mostnotably on his face and arms (Fig. 1). He also hadshallow erosions bilaterally on both shins (Fig. 2). No-table abnormal laboratory investigations included awhite blood count of 26.8 × 109/L and a normal neu-trophil count. The particular eosinophilia was 12 ×109/L. Lactate was 2.4 mmol/L and his albumin was31 g/L (total protein was 49 g/L). All other laboratorytests were normal. Skin swabs were taken of the ero-sions and a skin biopsy performed. The patient wasadmitted, started on intravenous vancomycin and clin-damycin and topical betamethasone 0.1% cream.Swabs taken from the shallow erosions eventuallygrew mixed Staphylococcus and Streptococcus species andhis biopsy showed subacute spongiotic dermatitis con-sistent with allergic contact dermatitis. He was there-fore diagnosed with erythroderma secondary to aller-gic contact dermatitis and started on oral prednisoneat a dose of 0.5 mg/kg/day.

DISCUSSION

Erythroderma typically presents with generalized ery-thema and scaling, classically affecting greater than90% of the total body surface area. Other manifesta-tions include diffuse alopecia, keratoderma, nail dystro-phy, ectropion, peripheral edema and lymphadenopa-thy.3 Symptoms generally include fevers, malaise,fatigue and pruritus. Significant systemic complicationsinclude fluid and electrolyte abnormalities, hypoalbu-minemia, thermoregulatory disturbance, cardiac failure,capillary leak syndrome, infection and possibly evendeath.4 There have been varied mortality rates from

Fig. 2. Shallow erosions on both of the patient’s shins.Fig. 1. Erythematous rash with desquamation diffusely onthe patient’s trunk, arms and face.

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Bruno and Grewal

246 2009; 11 (3) CJEM • JCMU

18% to 64% published, though mortality has been re-duced, generally because of advances in diagnosis andtreatment.5

Because of the extensive rash, fever, fatigue and po-tential for lymphadenopathy and weight loss, the pre-sentation of erythroderma can masquerade as, or be amanifestation of, more sinister conditions includinglymphoma (Sézary syndrome). Indeed, erythrodermamay be a presentation of reticuloendothelial or intravas-cular neoplasms, so it is incumbent on the physician toperform a thorough investigation. An algorithmic ap-proach to the erythrodermic patient begins with a thor-ough history, including a complete drug history. Thephysical examination requires specific attention to thevital signs, nails, mucosa and lymph nodes and the pos-sibility of hepatosplenomegaly. Baseline blood work andskin biopsies, along with a biopsy of enlarged lymphnodes, is usually the next step in the evaluation. Furtherinvestigations, such as chest radiography, patch testingand biopsies for immunofluorescence, can be carriedout as needed.6

Determining the underlying etiology and removingany contributing external factors remain the most im-portant factors in treating erythroderma.7 Erythro-derma is considered a dermatologic emergency; patientsshould be admitted to hospital promptly for treatment.Almost any drug can induce erythroderma; thereforeany potential offending agents should be stopped. Gen-eralized treatment of this condition includes hydrationto maintain a normal volume status, monitoring andcorrecting electrolyte abnormalities, keeping patientsafebrile and instituting gentle skin care measures. Forcrusted sites one can use oatmeal baths or wet dressingsfollowed by bland emollients and low potency topicalcorticosteroids.4 High potency topical corticosteroidsare generally avoided because of the possibility of in-creased systemic absorption due to the compromisedcutaneous barrier.8 Antihistamines can be used for theassociated pruritus. If there are any signs of secondaryinfection, systemic antibiotics should be started. If adrug reaction is suspected, oral corticosteroids at an ini-tial dose of 1–2 mg/kg/day may be initiated.1

CONCLUSION

Erythroderma is a complex disorder whose prognosisdepends on the etiologic cause. Virtually all cases ofdrug-induced erythroderma recover completely withprompt initial management and removal of the offend-ing drug. However, erythroderma can often be clini-cally mistaken for other presentations, such as an infec-tion, leading to higher mortality rates especially in thevery young and elderly. The attending emergencyphysician should always consider a broad differential di-agnosis when assessing a patient with possible erythro-derma. Individualized treatment based on the underly-ing etiologic cause should be combined with supportiveskin care to ensure optimal outcomes.

REFERENCES

1. Bolognia JL, Jorizzo JL, Rapini RP. Dermatology. 2nd ed.Toronto (ON): Mosby Elsevier; 2008. p. 149.

2. Karakayli G, Beckham G, Orengo I, et al. Exfoliative der-matitis. Am Fam Physician 1999;59:625-30.

3. Botella-Estrada R, Sanmartin O, Oliver V, et al. Erythro-derma: a clinicopathological study of 56 cases. Arch Dermatol1994;130:1503-7.

4. Rothe MJ, Bialy TL, Grant-Kels JM. Erythroderma. Derma-tol Clin 2000;18:405-15.

5. Sehgal VN, Srivastava G, Sardana K. Erythroderma/exfolia-tive dermatitis: a synopsis. Int J Dermatol 2004;43:39-47.

6. Rothe MJ, Bernstein ML, Grant-Kels JM. Life-threateningerythroderma: diagnosing and treating the “red man”. ClinDermatol 2005;23:206-17.

7. Wilson DC, Jester JO, King LE Jr. Erythroderma and exfo-liative dermatitis. Clin Dermatol 1993;11:67-72.

8. Aalto-Korte K, Turpeinen M. Quantifying systemic absorp-tion of topical hydrocortisone in erythroderma. Br J Derma-tol 1995;133:403-8.

Competing interests: None declared.

Correspondence to: Dr. Tony F. Bruno, Department of Family Medicine, CCFP-EM Program, Faculty of Medicine and Dentistry,205 College Plaza, University of Alberta, Edmonton AB T6G 2R3;tfbruno@shaw.ca

Acknowledgement: The authors would like to thank the De-partments of Family Medicine/Emergency Medicine and Der-matolgy for the funding contribution of this work.

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